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Congenital tumors of the umbilical cord are rare, and teratomas of the umbilical cord are exceedingly rare. Umbilical cord masses are often detected on routine prenatal ultrasound; however, umbilical teratomas are difficult to diagnose by imaging studies alone, and often require histopathologic examination for definitive diagnosis. Given the polymorphic presentation of umbilical cord teratomas and associated congenital anomalies, amniocentesis for genetic screening and fetal karyotyping should be offered. Here we report a case of a prenatally suspected umbilical cord angiomyxoma in an otherwise normal fetus who was found to be a mature teratoma on surgical pathology.